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Retinal Vasculitis

Reema Bansal MS and Amod Gupta MS

Advanced Eye Center, Post Graduate institute of Medical Education and Research,
Chandigarh
Email:eyepgi@sify.com

 

Retinal vasculitis is an inflammatory disease of the blood vessels of the retina that may be associated with primary ocular conditions or with inflammatory or infectious diseases in other parts of the body (systemic diseases). It has been defined as the vascular leakage and staining of vessel walls on fluorescein angiography, with or without the clinical appearance of fluffy, white perivascular infiltrates in an eye with evidence of inflammatory cells in the vitreous body or aqueous humor 1.

Typically, retinal vasculitis is divided into entities localized to the retina and into systemic diseases involving the eye. These include certain ocular disorders, systemic autoimmune disorders, and some infectious diseases.

Etiology:

The common primary causes of retinal vasculitis where vessel is the primary target of the inflammatory process are:

(a) Localized to the eye
                       
- Idiopathic
- Intermediate uveitis of the pars planitis type
- Frosted branch angiitis
- Idiopathic retinal vasculitis, aneurysms and neuroretinitis (IRVAN)

(b) Involving the eye and other organs (primary systemic associations)
                       
- Giant cell arteritis
- Takayasu arteritis
- Polyarteritis nodosa
- Wegener’s granulomatosis
                       

Churg-Strauss syndrome, Essential cryoglobulinemic vasculitis, and Cutaneous leucocytoclastic angiitis are some of the rare systemic associations.

The causes of secondary vasculitis (where vasculitis is a prominent feature but is secondary to an inflammatory process not primarily directed against the vessel) are:
           
(a) Localized to the eye:                    

(b) Associated with systemic involvement

Susac’s syndrome, Sjogren’s syndrome, Rheumatoid arthritis, Juvenile idiopathic arthritis, Whipple’ disease, and Rickettsial diseases are some of the rare systemic associations.

Clinical characteristics:

The classic symptom of retinal vasculitis is a painless decrease in vision. Other symptoms may include floaters from accompanying vitritis, a blind spot from ischemia-induced scotomas, metamorphopsia (change in shape of an object) in case of macular involvement or altered color perception. Retinal vasculitis can also be asymptomatic.

Retinal examination typically reveals sheathing (a whitish-yellow cuff of material surrounding the blood vessel) of the affected retinal vasculature associated with variable vitritis (inflammatory cells behind the lens in the vitreous body). It involves noncontiguous portions of the vessel. Inflammation may involve retinal arteries, veins or capillaries, but peripheral venous involvement is commonly recognized 2. Arterioles are preferentially involved in syphilis 3,4. The location and appearance of vascular lesions may have a limited diagnostic utility. "Candle- wax drippings" seen as dense, focal, nonocclusive periphlebitis are associated with sarcoidosis, but its appearance is neither pathognomonic nor present in all patients with the disease. The occlusive phlebitis of Behçet’s syndrome tends to manifest in the posterior pole, although peripheral retinal vasculitis may occur. Presence of a choroiditis lesion (active or healed) underlying the retinal vessels is a common observation in vasculitis of tubercular origin 5. Additional evidence of ocular inflammation such as cells in the aqueous humor may accompany retinal vasculitis.
Narrowing of the retinal blood vessels, vitreous hemorrhage, and new blood vessel growth are present as complications of retinal vasculitis.

 Diagnosis and testing:

The laboratory evaluation should be directed by a careful history and physical examination followed by targeted laboratory and radiological testing. Investigating a case of retinal vasculitis involves a tailored approach. Once a differential diagnosis is derived from a detailed history, ocular examination and a relevant systemic evaluation, only the specific tests are carried out. Random screening with full battery of tests is rarely productive and can be misleading.
Besides subjecting every patient of retinal vasculitis to fundus photography and fluorescein angiogram, we perform the following ancillary tests as and when indicated:

Few laboratory studies that should be done in all patients with isolated retinal vasculitis are:

When strongly suspecting a specific etiology, the following tests are ordered in relevance to the particular disorder:

A repeat evaluation and follow-up may be required in cases where laboratory test results do not yield any positive information. A large majority of cases still remain undiagnosed by the routinely available laboratory tests and continue to be labeled as idiopathic 2.
Certain tests such as liver function and renal function tests are ordered before initiating antimicrobial drugs or during the course of treatment before considering immunosuppressive therapy or while evaluating the adverse effects of some of the treating agents.

Management:

The treatment of retinal vasculitis depends on the detection of an associated condition, severity of the disease, and whether the process is unilateral or bilateral. The treatment of any type of uveitis has three main goals: to prevent vision-threatening complications, to relieve the patient's complaints and, when feasible, to treat the underlying disease6. Treatment of retinal vasculitis can be divided into three main aims: Suppression of inflammation, treatment of specific disease, if identified, and treatment of complications

 

Corticosteroids

They are the main stay of treatment in primary retinal vasculitis or those with underlying systemic vascular disease. Aggressive anti-inflammatory therapy is not indicated
for patients with asymptomatic vascular sheathing. In the absence of a specific infection, administration of corticosteroids helps to control the inflammation. Depending upon the severity and extent of the disease, oral prednisolone is started in a daily dose of 1 to 1.5mg/kg  body weight of the patient. As the inflammation subsides, tapering of corticosteroids by 5-10 mg per week is begun within 2-4 weeks of initiating therapy. Once the eye is completely quiescent, the corticosteroids are further tapered by 2.5-5 mg over few weeks and then discontinued.
Periocular depot steroids may be given as posterior sub-tenon injections in cases of unilateral disease. They are more commonly employed in our clinic for complications like cystoid macular edema.
The normal response to the corticosteroid therapy may be interrupted by recurrence of vasculitis in which case the oral corticosteroids are hiked to a dose of 1.5 to 2 mg/kg/day. Relapse of intraocular inflammation usually occurs when tapering is too rapid. All patients are monitored for side effects and complications of corticosteroids [secondary glaucoma, posterior subcapsular cataract, increased susceptibility to infection (ocular or systemic), hypertension, gastric ulcer, diabetes, obesity, growth retardation, osteoporosis and psychosis) at every follow up visit.

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